Unexplained sudden death remains one of the leading causes of death in sickle cell disease (SCD) adults. Ventricular arrhythmia is a well-known risk factor for sudden death but its prevalence and determinants in the context of SCD remain understudied.

From January 2019 to March 2021, consecutive adult patients with SCD referred to ambulatory cardiology department for possible cardiac involvement were prospectively included (Drepacoeur cohort). All patients that had 24-hour ECG monitoring (24h-holter) and thransthoracic echocardiography (TTE) on the same day were analysed. The primary end point was the occurrence of ventricular arrhythmia, defined as sustained or non-sustained ventricular tachycardia (VT), more than 500 premature ventricular contractions (PVC) on 24h-holter, or history of VT ablation.

Overall, 90 patients were included and 54 (60%) were analysed. Mean age was 47.6±11.6 years (range 21-69), 53% were male. Heart function was mainly preserved with a mean left ventricular ejection fraction (LVEF) of 57.9±4.9% and a mean global longitudinal strain (GLS) of -18±2.8%. Mean tricuspid regurgitation velocity was 2.6±0.4m/s. Ventricular arrhythmia was observed in 13 (24.1%) patients (4 non-sustained VT, 9 with more than 500 PVC and 1 history of VT ablation). Ventricular arrhythmia was associated with lower GLS (−15.8±1.8% vs. −19±2.7%, P<0,001), lower tricuspid annular plane systolic excursion (TAPSE, 23±5.7mm vs. 26.9±4.6mm, P=0,02) and more dilated right ventricle assessed by tricuspid annulus diameter (38.2±6.1mm vs. 34.2±4.5mm, P=0,02). In multivariate analysis, GLS was independently associated with ventricular arrhythmia (OR=2,1 CI95% [1,3; 3,3], P=0,004) with a moderate exponential correlation with PVC load on 24h-holter (R=0,5, P<0,001).

In SCD adults with preserved LVEF, GLS was the only independent echocardiographic predictor of ventricular arrhythmia.